Khalifa Mabrouk, Laatiri Mohamed Adnène, Chehata Sami, Rhaiem Kaief, Gharbi Olfa, Amama Wafa, Ennabli Souad
Service d'Hématologie Clinique, CHU F. Hached de Sousse, Tunisie.
Tunis Med. 2003 Apr;81(4):226-9.
Between 1989 and 1999, 36 cases with primary myelodysplastic syndromes were diagnosed. They were 15 male and 21 females, the median age was 62 years (range: 22 to 90 years). Eighty one per cent of patients were presented symptoms of anemia. Lymphadenopathy, splenomegaly and skin manifestations were noted in 25% of cases. Hemogram showed anemia, leucopenia and thrombocytopenia respectively in 97%, 44% and 55% of cases. Refractory anemia with excess blasts (AREB) is the most frequent FAB subtypes of MDS (17 cases). Cytogenetic study concerned 24 patients. In 13 cases the karyotype was pathological with deletion 5 q in 64% of cases. Seventeen patients have received a chemotherapy. Survival rate to 36 months is 11%. At the time, the only curative treatment is the bone marrow transplantation, which is proposed to young patients with HLA identical donor.
1989年至1999年间，共诊断出36例原发性骨髓增生异常综合征患者。其中男性15例，女性21例，中位年龄为62岁（范围：22至90岁）。81%的患者出现贫血症状。25%的病例出现淋巴结病、脾肿大和皮肤表现。血常规显示，97%、44%和55%的病例分别出现贫血、白细胞减少和血小板减少。难治性贫血伴原始细胞增多（AREB）是骨髓增生异常综合征最常见的FAB亚型（17例）。24例患者进行了细胞遗传学研究。13例患者的核型异常，其中64%的病例存在5号染色体长臂缺失。17例患者接受了化疗。36个月的生存率为11%。目前，唯一的治愈性治疗方法是骨髓移植，适用于有HLA相合同胞供者的年轻患者。
